Abdominal tuberculosis: CT evaluation

The computed tomography (CT) scans of 27 patients with abdominal tuberculosis were reviewed retrospectively to determine the range of abdominal involvement. Most patients had been at increased risk because of intravenous drug abuse, alcoholism, acquired immunodeficiency syndrome (AIDS), cirrhosis, or steroid therapy. The etiologic agent was Mycobacterium tuberculosis in 23 patients and M. avium-intracellulare in four patients with AIDS. In five patients, tuberculosis was limited to the abdomen. CT findings included adenopathy, splenomegaly, hepatomegaly, ascites, bowel involvement, pleural effusion, intrasplenic masses, and intrahepatic masses. Characteristic features were a tendency for adenopathy to prominently involve peripancreatic and mesenteric compartments, low-density centers within enlarged nodes, complex nature of the ascites, and adenopathy adjacent to sites of gastrointestinal tract involvement. Recognition of these manifestations and maintenance of an index of suspicion, especially in patients at risk, should help optimize the correct diagnosis and management of intraabdominal tuberculosis.     

Livedo reticularis associated with interferon α therapy in two melanoma patients

We report two patients who developed intense livedo reticularis clearly related to the administration of interferon alpha 2b as an adjuvant therapy for melanoma. Histological studies showed scattered perivascular infiltrates without vasculitis. Laboratory tests excluded any underlying condition. Resolution of the symptoms was observed in both patients when interferon alpha was withdrawn. These cases highlight the occurrence of livedo reticularis as an uncommon side-effect of interferon alpha treatment.     

Endothelial markers and homocysteine in patients with classic Fabry disease

Aim : Fabry disease is an X-linked inborn error of glycosphingolipid metabolism due to the deficient activity of α-galactosidase A, a lysosomal enzyme. It is a multisystem disorder characterized by progressive renal insufficiency, with added morbidity from cardio- and cerebrovascular involvement. The recent availability of genetically engineered enzyme offers an effective targeted treatment approach, but also emphasizes the need for surrogate markers to delineate organ damage and monitor the efficacy of enzyme replacement therapy (ERT). Methods : Multiple endothelial factors and plasma homocysteine concentrations were investigated in 12 consecutive hemizygous males with classic Fabry disease and 15 controls as part of an exhaustive baseline evaluation prior to ERT. Results : Compared with the controls, plasma concentrations of homocysteine were significantly ( p > 0.01) higher in patients with Fabry disease in the absence of chronic renal failure or vitamin deficiency. Plasma concentrations of vascular cell adhesion molecule-1 were also significantly ( p > 0.05) higher in the patients, and there was a trend for decreased endothelin-1 levels. No difference was found in serum intercellular adhesion molecule-1, plasma P-selectin, serum E-selectin and plasma thrombomodulin between the patients and controls.
Conclusions : The results do not reveal measurable evidence for endothelial and leukocyte activation that could reliably serve as surrogate markers for routine monitoring of the efficacy of ERT in patients with Fabry disease. While the exact origin and clinical significance of hyperhomocysteinaemia in Fabry disease remains to be studied in a larger cohort of patients carefully monitored for their concurrent medications, especially carbamazepine, we suggest that patients may benefit from folic acid or multivitamin therapy to treat this additional vascular risk factor, when present.     

Hypoxaemia in infants with respiratory tract infections

Nineteen infants who were graduates from special care baby units underwent two overnight tape recordings of oxygen saturation (SaO2) and breathing movements; one during an upper (n = 12) or lower (n = 7) respiratory tract infection and the other when free of infection. Baseline SaO2 was lower during infection (median 99.6 vs 100%, p less than 0.01), with four patients having values (84.3-95.5%) below the normal lower limit for full-term infants (97%). The median number of apnoeic pauses was also lower during respiratory tract infection (4.7 vs 15.7/h, p less than 0.02). The median number of episodic desaturations (SaO2 less than or equal to 80%) did not change significantly (1.3 vs 1.9/h, p greater than 0.05), with the exception of one patient who had extremely increased values during infection for both apnoeic pauses (63/h) and desaturations (112/h). No infant, however, was considered clinically hypoxaemic. Clinically unsuspected hypoxaemia may thus occur during respiratory tract infection in a proportion of infants graduating from special care baby units. Such hypoxaemia may have potentially deleterious effects.